[Severe childhood atopic dermatitis]. / La dermatite atopique severe de I'enfant.

BACKGROUND: Atopic dermatitis (AD) is a chronic relapsing eczematous skin disease. It represents one of the symptoms of atopic diathesis. DA affects usually infants and children. aim : The aim of our study is to draw up the epidemiological, clinical features, treatment and outcome of severe childhood AD through a hospital series. methods: A retrospective study of 24 cases of severe childhood AD hospitalized in the Dermatology Department of La Rabta hospital of Tunis was conducted during a 28 year-period (1981 - 2009). results: The hospital incidence of severe childhood AD was 0,085‰. Patient's mean age at the beginning was 14 months. The sex ratio H/F was 1.66. Cutaneous manifestations occurred preferentially in face (75%). Generalized eczema was observed in 37.5% of cases. Pruritus and xerosis were constant. The mean duration of hospitalization was 11 days. Topical corticosteroids was the most effective method of treating severe DA, associated with antiseptic solutions emollient and antihistaminic drugs. Infectious complications were noted in 50% of cases. Ocular complications were observed in 16.7% of cases. Recurrences were reported in 9 cases. Conclusion :AD is an inflammatory, chronically relapsing, and pruritic skin disorder developing in a xerotic skin. Severe AD in childhood is rare in Tunisia. It requires a good understanding of therapeutic modalities by the patient and his family. It is a cause of important morbidity and it may have a bad impact on quality of life.

Lichen planus pemphigoides: four new cases and a review of the literature.

Lichen planus pemphigoides (LPP) is a rare autoimmune blistering disease. It appears to be combination of lichen planus and bullous pemphigoid. We describe four new cases of LPP and discuss the epidemiological, clinical, pathological, and therapeutic features of this singular association through a review of the 74 published cases within the English literature. We report four cases of LPP (three women aged respectively 47, 51, and 53 years old, and a 53-year-old man). All patients presented with bullae on lichenoid and normal skin, predominately on the extremities. The diagnosis was confirmed by immunohistological findings. Our patients were treated with oral corticosteroids with a good response. Our review of the literature of 78 cases of LPP (65 adults and 13 children) showed that it involved adults (mean age: 54 years), with a slight female preponderance. A mean lag time between LP and the development of LPP was 8.3 months. LPP is characterized by developing blisters on lichenoid lesions and on uninvolved skin with more acral distribution of bullous lesions. Involvement of palms and soles was more frequent in children. The diagnosis is based on pathological and immunological confrontation. LPP is usually idiopathic, but some cases were reported in association with various drugs. There have also been reports of association with internal malignancy. Most cases of LPP are successfully treated with systemic corticosteroids. In most cases, the prognosis was good.

[Kindler syndrome: clinical and ultra-structural particularities, a propos of three cases]. / Le syndrome de Kindler, particularités cliniques et ultrastucturales à travers trois nouvelles observations familiales.

INTRODUCTION: Kindler's syndrome is a rare type of genetic skin condition belonging to the class of bullous poikilodermia. We report three new sibling cases of this rare syndrome. CASES REPORTS: The condition was seen in three sisters aged 12, 16 and 20 years, born of a first-degree consanguineous marriage with no family history of Kindler's syndrome. The three patients presented spontaneously regressive bullous eruptions, poikilodermia of gradual onset, major cutaneous atrophy on the back of the hands and the feet, photosensitivity and gingival hypertrophy. Electron microscopy examination of poikilodermic skin showed normal anchoring filaments and intraepidermal cleavage. DISCUSSION: Diagnosis of Kindler's syndrome is based upon clinical evidence. Kidler's syndrome is a well defined clinical entity. Ultra-structural studies show intraepidermal, junctional, and dermal cleavage. This syndrome must be differentiated from congenital epidermolysis bullosa, Weary's syndrome, and other bullous hereditary poikilodermas.

[Hyalinosis cutis et mucosae : 5 cases from Tabarka (Tunisia)]. / La hyalinose cutanéo-muqueuse : 5 cas originaires de Tabarka (Tunisie)

BACKGROUND: Hyalinosis cutis et mucosae (HCM), is a rare autosomal recessive genodermatosis. Cutaneous features are characteristic and allow to suspect diagnosis. AIM: To report a series of HCM. METHODS: A retrospective study of all cases of HCM, diagnosed in a dermatology department over a period of 25 years (1983-2007). RESULTS: Over the considered period, 5 new cases of HCM were diagnosed. Patients were aged between 14 and 41 years. They were 3 females and 2 males. All patients were native of Tabarka (northwestern Tunisia). The age of the onset of the disease varied from neonatal period to 5 years. Hoarseness was the first clinical manifestation in all cases. Skin lesions developed between the ages of 3 and 8 years. Vesiculobullous lesions were observed in 2 patients. Moniliform blepharosis was seen in all patients. Warty and hyperkeratotic papules were observed in 3 patients. Diffuse thickening of the skin was seen in 3 patients. Lesions were primarily distributed on the face. All patients presented diffuse scars. Linear palmoplantar keratoderma was seen in one patient. Asymptomatic endocranial calcifications were noted in 4 patients. A pituitary adenoma was noted in one patient. Histopathological examination of a skin lesion revealed a typical pattern of HCM. Two patients were treated with systemic retinoids without improvement. CONCLUSION: We reported five new cases of HCM. All patients were native from Tabarka. We report also one case of linear palmar keratoderma associated with HCM. This association was not reported in the literature. Finally, the association HCM-pituitary adenoma, seen in one patient, may be fortuitous.

[Chronic lupus erythematosus: 104 Tunisian cases]. / Le Lupus Erythémateux Chronique: 104 cas tunisiens.

BACKGROUND: Chronic lupus erythematosus is a cutaneous form of lupus erythematosus, usually involving photoexposed areas. AIM: The aim of our study is to evaluate the epidemio-clinical trends, therapeutic features and outcome of patients with chronic lupus erythematosus, through a Tunisian hospital series. We have tried to compare our results with those of other African and western series. METHODS: It is a retrospective study, concerning all patients with chronic lupus erythematosus, attending the Dermatology department of Charles Nicolle Hospital over an 11 years period. The patients' age, sex, clinical features, explorations results, treatment and evolution were recorded. RESULTS: A total of 104 patients were included. Chronic lupus erythematosus represented 0,1 % of all the dermatitis seen over 11 years. Chronic lupus erythematosus affects young women with a ratio F/M of 1,97 and an average age of 42 years. The discoid form was the most frequent clinical shape, observed in 73 % of cases (76 patients). The face was the most frequent localization of the lesions (91%). Five patients (4.8%) presented a generalized chronic lupus erythematosus. The direct immunofluorescence in sick skin was positive in 54 % of cases. Treatment included sun avoidance and oral antimalarials drugs in the majority of cases (95%). The progression from chronic lupus erythematosus to systemic lupus erythemetosus was observed in two cases (2%). CONCLUSION: Chronic lupus erythematosus is a relatively rare disorder in Tunisia, compared to other African countries, where prevalence is 7 to 10 times more frequent. As shown in our study, the most common clinical feature is the discoid form. The diagnosis can be confirmed by histopathological and direct immunofluorescence examination. Progression of chronic lupus erythematosus to systemic lupus erythematosus is possible, as observed in two of our patients. Thus, Patients with chronic lupus erythematosus should be continually followed up.